Hypothalamic hamartomas: A comprehensive review of the literature – Part 1: Neurobiological features, clinical presentations and advancements in diagnostic tools

Safwan O. Alomari, Mohammed N.El Houshiemy, Shadi Bsat, Charbel K. Moussalem, Mohammed Allouh, Ibrahim A. Omeis

Research output: Contribution to journalReview articlepeer-review

2 Citations (Scopus)

Abstract

Hypothalamic hamartomas (HH) are rare, non-neoplastic heterotopic tissues which contains normal neurons and glia including oligodendrocytes and fibrillary astrocytes but in an abnormal distribution. They arise from the floor of the third ventricle, tuber cinereum, or mammillary bodies. Estimated incidence ranges from 1 in 50,000–1 in 1,000,000. Hypothalamic hamartomas are associated with different clinical presentations including various types of seizures, most characteristically; the gelastic seizures, precocious puberty, cognitive impairment, and behavioral changes. In this review, the authors discuss advancements in different diagnostic elements of hypothalamic hamartoma; including clinical features, EEG findings, and neuroimaging techniques. Moreover, different classifications described in the literature will be discussed.

Original languageEnglish
Article number106076
JournalClinical Neurology and Neurosurgery
Volume197
DOIs
Publication statusPublished - Oct 2020

Keywords

  • Gelastic seizures
  • Hypothalamic hamartoma (HH)

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

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